Elwood, the Chinese Crested and Chihuahua mixed breed shot to prominence after winning the World's Ugliest Dog Contest in 2007.
He died on Thanksgiving morning, said its owner Karen Quigley from New Jersey.
"I couldn't be more proud that people who haven't met him loved him ... I couldn't be more proud," Quigley was quoted by CNN as saying.
Elwood was only two-years-old when people outside southern Jersey first saw his distinctive mug.
His looks landed him the title of the World's Ugliest Dog as well as fans from Brazil to Germany and beyond at a contest held in California.
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Quigley was so inspired by the story of Elwood, that she wrote a children's book about him called "Everyone Loves Elwood."
"For a little dog he touched a lot of lives in his short time. He did a lot of things," said Quigley.
Elwood passed away unexpectedly last week. Quigley said she didn't know the cause of death, only that he had recently fallen ill.